颅骨骨纤维异常增生症的诊断及治疗

骨纤维异常增生症(fibrous dysplasia,FD)又名骨纤维结构不良症,根据其临床表现有不同的分型.其中颅骨FD主要的临床表现是视力障碍.治疗视力障碍的方法是视神经管减压,但手术时机及术后疗效国内外均有不同报道.我院自2003年1月至2009年6月共对27例颅骨FD患者进行视神经管减压治疗,术后疗效较好,现报告如下.     

经眶外侧颅内外联合径路治疗颅眶骨纤维异常增殖症

目的 探讨颅眶骨纤维异常增殖症手术治疗的径路. 方法 对累及额骨、眼眶、蝶骨、颞骨的复杂颅面骨纤维异常增殖症患者,应用经眶外侧颅内外联合径路进行手术治疗,包括眶外侧及颞部开窗,病灶的部分或大部切除,视神经减压,颅眶骨外形重建等. 结果 自2005年以来,于临床治疗8例患者,术后随访9个月至3年,无明显并发症发生,复杂颅眶部畸形得以矫正,视力障碍有所改善.头颅CT示骨瓣愈合良好,未见病变复发. 结论 经眶外侧颅内外联合径路可作为治疗颅眶部骨纤维异常增殖症的首选方法.     

颌面部骨纤维异常增殖症围手术期护理体会

目的对16例颌面部骨纤维异常增殖症患者术后围手术期护理进行总结。方法自2007年9月至2020年9月,对16例颌面部骨纤维异常增殖症患者采用专科护理评估、围手术期护理及心理护理相结合的综合护理方法,术后随访6个月,评价患者护理满意度及术后并发症。同时选取自2000年9月至2007年9月,收治的16例颌面部骨纤维异常增殖症患者进行比较。结果与对照组相比,本组16例患者均未出现严重的并发症,效果较满意。结论对临床采用手术治疗的颌面部骨纤维异常增殖症的患者,在手术治疗的同时配合细致的综合护理方法,不但能减少手术并发症的发生,还有利于患者面部功能及外形的恢复,值得临床上参考。     

经颅硬膜外入路双层壁视神经管减压术的研究

目的 探讨经颅硬膜外入路治疗双层壁视神经管视神经损伤中的手术方法及效果.方法 选择6例经CT证实双层壁视神经管的视神经损伤患者,经颅硬膜外入路,切除气化筛窦构成的眶上壁至视神经管眶口,显微镜下切除气化的视神经管上壁,切断筛后动脉,自额筛缝之前咬除眶尖骨质及视神经管上壁、内侧壁和外侧壁,减压周径大于1/2而小于3/4,未剪开视神经鞘,封闭蝶窦及后组筛窦的漏口.结果 6例患者均有效,其中3例视力达0.1及以上,无脑脊液漏,颅内感染.结论 经颅硬膜外入路双层壁视神经管减压术是针对视神经管变异为双层时的有效治疗,具有减压范围充分的特点.视神经损伤患者为双层壁视神经管时应积极手术治疗.     

"狮面人"畸形矫治术麻醉处理1例

患者，男性，28岁，身高172cm，体重54kg。缘于20年前无诱因左额部出现一包块，逐渐增大，波及左眼睑、额顶、面部、腮部，巨大肿物范围后至顶枕，右至顶右眶鼻，左至颢顶左耳腮到下颌，左眼球突出，睁眼受限，左鼻孔明显扩大，左上唇口角受累，左上颌骨变形，颅面部呈“狮面状”。8年前行增生组织部分切除术，近2年增长加快收入院，初步诊断：颅面部巨大肿物，骨纤维异常增殖症伴胶原纤维增生。     

计算机导航技术在眶颧部骨纤维异常增生症治疗中的应用

目的探讨计算机导航技术在眶颧部骨纤维异常增生症手术中的应用价值及疗效分析。方法以2016年3月至2018年12月上海交通大学医学院附属第九人民医院整复外科收治的眶颧部骨纤维异常增生症患者为研究对象,均行术前三维CT扫描及计算机辅助设计,应用镜像设计原理将健侧与患侧进行配对比较,确定术中异常骨质切除范围并标明术中危险区域。术中将导航支架置于患者前额,配对完成后,于计算机导航辅助下经下睑缘-口内切口入路根据术前方案行异常骨纤维切除。于术前、术后6个月头颅CT三维重建模型上标记颧颌点、眶下点、颧突点及颧颌缝眶下缘点,分别测量其到正中矢状面的距离,采用配对t检验分别对患者术前和术后健、患双侧对称性进行分析。术后对眶颧部形态进行对比,并统计并发症发生情况。结果共纳入16例眶颧部骨纤维异常增生患者,其中男性12例,女性4例,年龄18~35岁。术前健、患双侧颧颌点、眶下点、颧突点到正中矢状面距离比较,差异均有统计学意义(P<0.01);颧颌缝眶下缘点双侧比较,差异无统计学意义(P>0.05)。术后健、患双侧4个标志点到正中矢状面的距离比较,差异均无统计学意义(P>0.05),健、患侧颧颌点到正中矢状面距离的差值由(10.1±6.2) mm减少至(2.7±6.4) mm,眶下点由(7.7±4.6) mm减少至(1.9±3.9) mm,颧突点由(8.5±7.5) mm减少至(0.7±1.5) mm,颧颌缝眶下缘点由(1.9±3.5) mm减少至(0.6±2.1) mm。所有患者术后疗效显著,患侧与健侧形态对称,轮廓自然,无并发症发生,满意度较高。结论应用计算机导航技术治疗眶颧部骨纤维异常增生症可有效解决经下睑缘-口内入路视野暴露困难的问题,显著提升手术的精确性、对称性及安全性。     

L形截骨颧骨降低术治疗颧上颌复合体骨纤维异常增殖症

目的：评价L形截骨颧骨降低术治疗颧上颌复合体骨纤维异常增殖症的效果。方法：颧上颌复合体骨纤维异常增殖症致面中份畸形13例，颧上颌复合体不同程度向前外侧突出，经下睑缘及口内切口入路，在切除上颌骨、颧骨以及眼眶内大部分增生骨质的同时，行L形截骨颧骨降低，小夹板坚强内固定术。结果：随访6～12月，无复发。13例患者的外形均取得满意的治疗效果，功能没有障碍。结论：L形截骨颧骨降低术治疗颧上颌复合体骨纤维异常增殖症，是除美容整形外的又一推广应用，疗效可靠。     

面部肥厚型鲜红斑痣的手术治疗

目的 总结头面部肥厚型鲜红斑痣的治疗经验,探讨其手术方式的选择及疗效.方法 自1990年1月至2010年12月,共收治126例头面部肥厚型鲜红斑痣患者,采用单一术式或联合多种术式进行个性化治疗.其中,行选择性切除者31例;完整切除+分区植皮者59例;选择性切除+局部皮瓣+分区植皮者13例;完整切除+局部皮瓣+分区植皮者23例.结果 本组126例患者中,术后切口感染者3例,切口裂开者1例,皮片部分坏死者9例,局部复发者14例,均经对症处理后患者满意.局部皮瓣完全成活.术后随访所有患者3个月至2年,效果均较满意.结论 根据患者的病变特点选择相应的手术方式,可使面部肥厚型鲜红斑痣的治疗取得良好的效果.     

经颅显微减压术治疗创伤性视神经损伤

目的探讨经颅入路视神经减压术的手术方法和显微手术技巧,以提高创伤性视神经损伤的手术疗效。方法行减压术治疗创伤性视神经损伤36例,取冠状切口骨瓣开颅（或翼点）入路,在手术显微镜直视下手术,切开颅底硬脑膜,暴露及开放视神经管上壁和外侧壁,显露视神经不少于1/2周径,剪开视神经鞘膜和总腱环。结果本组均术后随访3月以上,28例视力明显提高,7例术后视力未恢复,1例术后视力恶化,无术后并发症。结论适宜的手术时机、合理的手术入路、充分的术中减压和精细的显微外科技巧可显著提高创伤性视神经损伤的手术疗效。     

同种异体骨移植修复骨纤维异样增殖症术后骨缺损

目的观察同种异体皮质骨移植可吸收钉固定修复骨纤维异样增殖症刮除术后皮质骨缺损的疗效。方法自2004年3月至2007年4月我科共收治骨纤维异样增殖症24例,其中股骨11例,肱骨5例,合并桡骨2例,股骨颈及转子间1例,胫骨6例（合并腓骨1例）,单纯腓骨1例。24例中6例出现病理骨折并骨愈合。所有患者入院后行病灶刮除,松质骨打压植骨后运用同种异体皮质骨移植可吸收螺钉固定修复皮质骨缺损。术后1、2、3、6、9、12个月对患者进行门诊随访,此后每年随访一次,观察患者的移植骨愈合、并发症及患肢功能恢复情况。结果术后患者切口均Ⅰ/甲级愈合。术后1年,24例患者植入的骨粒及皮质骨块与正常骨质均基本融合;术后1年3例失访,随访的21例术后2～5年移植骨吸收重建,骨缺损修复,病灶消失,其中14例骨干病灶中有6例髓腔再通。3例残留手术区域隐痛,与天气变化有关。所有患者截止随访末期功能均良好,无复发。1例患者术后3年邻近腓骨出现新病灶,5年后因摔伤导致腓骨病理性骨折,但胫骨无骨折。结论同种异体皮质骨移植可吸收钉固定修复骨纤维异样增殖症刮除术后皮质骨缺损疗效可靠。     

颅面骨纤维结构不良致视神经管狭窄的手术治疗

目的 探索颅面骨纤维结构不良所致的视神经管狭窄的手术治疗策略。方法 回顾性分析2012年8月至2017年9月收治的18例颅面骨纤维结构不良所致的视神经管狭窄的手术病例,总结分析其临床表现、影像学表现、手术方法和结果。结果 7例患者接受治疗性视神经管减压,5例患者接受预防性视神经管减压,6例患者单纯接受骨纤切除整复。随访结果显示,12例行视神经管减压手术患者中,3例视力改善,7例保持稳定,2例视力恶化。结论 对于颅面骨纤维结构不良导致的视神经管狭窄、视力障碍,视神经管减压手术能有效终止视力恶化趋势,并有改善视力的可能。使用神经导航技术进行术前设计与术中辅助,可有效提高手术效率及安全性,降低视神经损伤、视力恶化风险。     

Optic Nerve Decompression for Orbitofrontal Fibrous Dysplasia

Orbitofrontal fibrous dysplasia often involves the bony orbit and the optic canal. Although fibrous dysplasia reportedly produces compression of the optic nerve leading to visual distrubances, optic nerve decompression in patients without clinical signs of optic neuropathy is still controversial. We describe two patients with orbitofrontal fibrous dysplasia without signs of visual disturbance and one patient with McCune-Albright syndrome and progressive visual impairment. Optic nerve decompression was performed prophylactically for two patients and therapeutically for one patient through the transcranial extradural route. Dystopias and craniofacial deformities induced by fibrous dysplasia also were corrected. The micropressure suction-irrigation system was especially effective for decreasing heat transfer and thereby preventing thermal injury of the optic nerve. The orbitofrontal area was reconstructed from cranial bone, iliac bone, and ribs. Postoperative follow-up revealed no disturbances in visual function and no evidence of cerebrospinal fluid leakage. These findings suggest that optic nerve decompression may be effective in preventing visual disturbances with minimal risk of other neurological sequelae. Subsequent orbital reconstruction yielded satisfactory cosmetic results.     

Bladder neck contracture following prostatectomy

In an 11-year period, 49 patients developed bladder neck contracture after prostatectomy--an incidence of 0.86%. This complication was found to be more common after resection of small fibrous hyperplastic prostates. The best results for treatment of the contracture were obtained after bladder neck incision, which gave a 9% incidence of recurrence; after transurethral resection of the bladder neck contracture the recurrence rate was 46%, and after bladder neck dilatation it rose to 100%.     

Optic nerve decompression for orbitofrontal fibrous dysplasia: recent development of surgical technique and equipment.

OBJECTIVES: Orbitofrontal fibrous dysplasia often involves the bony orbit and optic canal. Although fibrous dysplasia reportedly produces compression of the optic nerve leading to visual disturbances, optic nerve decompression in patients without clinical signs of optic neuropathy remains controversial. We describe the recent development of surgical techniques and equipment for optic nerve decompression in orbitofrontal fibrous dysplasia. METHODS: Optic nerve decompression was performed prophylactically for five patients and therapeutically for one patient using the transcranial extradural route. A high-speed drill and continuous suction-irrigation system has been used in five patients since 1998, and an ultrasonic bone curette in two patients since 2004. RESULTS: The continuous suction-irrigation system was particularly effective for decreasing heat transfer and thus preventing thermal injury to the optic nerve from the high-speed drill. The ultrasonic bone curette was also effective, allowing bone removal with minimal pressure from the tip of the handpiece and without catching cotton pledgets or damaging surrounding tissues. Orbital dystopias and craniofacial deformities induced by fibrous dysplasia were also successfully corrected. Postoperatively, disturbance in visual function was present in only two patients. Mean follow-up period was 4.9 years. CONCLUSIONS: This equipment may contribute to the development of new modalities for optic nerve decompression in orbitofrontal fibrous dysplasia.     

The surgical treatment of fibrous dysplasia. With emphasis on recent contributions from cranio-maxillo-facial surgery.

Fibrous dysplasia is a congenital, metabolic, nonfamilial disturbance that occurs in one or more bones, at times in association with skin pigmentations or endocrine abnormalities. The authors report on a large personal series of 23 patients with fibrous dysplasia involving the craniofacial skeleton. The etiology, clinical findings, pathology, and differential diagnosis of this condition are reviewed and a working hypothesis is offered for the pathophysiology of this disorder. Approximately one-third of patients with fibrous dysplasia have involvement of the cranial or facial bones. The authors describe how new techniques in craniofacial surgery have opened up additional options for this group of patients. Deformity, diplopia, proptosis, sinus infection, deafness, and loss of vision, are some of the clinical features that may require early surgical management. Evidence is given to support more complete resection of bony lesions with immediate reconstruction by several techniques. The removal, remodeling, and replacement of the dysplastic bone is advanced as a promising new method for the management of these complex problems. Successful use of this technique in four patients is reported. In a separate group of patients, continuing good experience is reported with cranio-orbital reconstruction by means of large methyl-methacrylate implants. Both of these surgical approaches eliminate donor site morbidity that results from the grafting of large amounts of autogenous bone. Both techniques also avoid the problems associated with postoperative absorption of bone grafting. Several patients are reported in whom serious disturbances in visual function appear to have been prevented or reversed by early treatment. Factors leading to malignant change in patients with fibrous dysplasia are reviewed.     

Bilateral proptosis: an unusual presentation of fibrous dysplasia

Fibrous dysplasia is a benign, developmental bone disorder of unknown aetiology. With involvement of the facial bones, indications for surgical intervention include cosmetic deformity and/or progressive neurological dysfunction. We present the case of a 36-year-old female with bilateral orbital lesions whose initial presentation was that of progressive proptosis and visual loss. She underwent bilateral pterional craniotomies with gross total resection of her tumours. Review of the literature reveals several cases of unilateral orbital fibrous dysplasia. This appears to be the first reported case of distinct, bilateral orbital fibrous dysplasia.     

Surgical reconstruction of fibrous dysplasia of bone in long-term follow-up

PURPOSE: To examine how different operative measures influence the surgical outcome in patients with fibrous dysplasia of bone. METHODS: 118 dysplastic fibrous lesions of bone were surgically treated and reviewed in 70 patients between 1983 to 1993 (eleven years) with a median follow-up of six and a half years. Surgery consisted of intralesional curettage in 93 and marginal en bloc resection in 25 lesions. Bony defects were reconstructed with autogenous iliac crest graft in 55 lesions, with autogenous fibula graft in 9, with homologous bone chips in 28, and 5 times with a homologous fibula graft from the bone bank. In 33 lesions the entire defect was filled with polymethylmethacrylate. Osteosynthesis was performed in 41 patients. RESULTS: Recurrences requiring surgical revision were observed in 26 of 74 primary lesions (= 35% overall recurrence rate) at a mean 123.6 weeks postoperatively. The most frequent primary and recurrence location was the proximal femur (85% revision rate). 69% of all recurrences occurred under the age of 20. After intralesional curettage the reoperation rate was 32% and after marginal resection 8%. After reconstruction with autogenous iliac crest graft recurrence rate was 36%, after autogenous fibula graft 55%, after homologous bone chips 18%, after polymethylmethacrylate 9% and allograft fibula reconstruction showed no recurrences. A combined stable osteosynthesis bridging the fibrous osseous defect significantly reduced the revision rate to 3% (p = 0.01). CONCLUSION: Intralesional curettage and reconstruction with autogenous iliac crest graft in fibrous dysplasia of bone leads to a high recurrence rate. Reconstruction with cortical grafts or bone chips from the bone bank, if necessary in combination with a durable osteosynthesis in mechanically demanding locations, or solely bone cement in mechanically less demanding areas, reduces the revision rate in patients with monoostotic and polyostotic fibrous dysplasia.     

A clinicoradiologic analysis of symptomatic craniofacial fibro-osseous lesions

OBJECTIVE: To outline the clinical and radiologic aspects of symptomatic craniofacial fibro-osseous lesions and to study the appropriate surgical management with follow-up results. STUDY DESIGN AND SETTING: A retrospective review of 11 patients who underwent surgical treatment during 1985 to 2004 in a tertiary health care center. RESULTS: Most (72.7%) cases were of fibrous dysplasia and were under 25 years of age (72%). Maxilla was the most common bone involved (81%). Lateral rhinotomy was the main approach for surgical resection. One patient underwent bilateral optic nerve decompression (left side therapeutic and on right side prophylactic). Six (54.5%) cases had recurrences that were managed by either radical surgery (total maxillectomy) or by further shaving off the lesion. CONCLUSION: Treatment of craniofacial fibro-osseous lesions is highly individualized. A conservative approach may not be able to treat all cases of craniofacial fibro-osseous lesions. A more radical approach that includes a craniofacial resection or a total maxillectomy may be warranted in few cases.     

Monostotic fibrous dysplasia of the cranial vault: a case report

Fibrous dysplasia, when it occurs in the craniofacial region, mostly involves the skull base and is rarely localized in the cranial vault. Although there have been several reports on magnetic resonance imaging (MRI) findings of fibrous dysplasia involving the skull base, cases occurring in the cranial vault have seldom been reported. We describe here a rare case of monostotic fibrous dysplasia that occurred in the parietal bone and discuss the characteristics of the MRI findings. A 47-year-old female was admitted to our hospital with a complaint of vertigo. A computed tomography (CT) scan did not reveal any intraparenchymal lesions in either the infra- or supratentorium, and her vertigo improved immediately without any treatments. However, a solitary osteolytic lesion was found incidentally in the left parietal bone. MRI showed that the lesion demonstrated hypointensity on T1-weighted images and hyperintensity on T2-weighted images, and was enhanced heterogeneously following injection of Gadolinium-DTPA. Removal of the parietal bone containing the lesion was performed according to the patient's wishes. The histopathological findings of the removed tissues corresponded to fibrous dysplasia. Although it is well known that craniofacial fibrous dysplasia demonstrates iso- or hypointensity on T1- as well as T2-weighted images, in the present case, the lesion showed apparent hyperintensity on T2-weighted images. These findings suggest that fibrous dysplasia can display various MR intensities depending on its origin.